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CŒLIAC DISEASE AND MALIGNANT CHANGE
544 brain
compression and raised intracranial pressure. Vomiting, convulsions, changes in muscular tone, and loss of consciousness force the parents to seek medical aid. Important physical signs are retinal or subhyaloid haemorrhages on ophthalmoscopic examination. When the limbs have been damaged, the baby has painful, tender swellings of the arms and legs, with or without cutaneous bruising. His general state is often good, and this, together with the absence of a history of trauma, misleads the doctor into believing that an acute natural disease is the cause of the trouble. Suspicions will be more readily aroused if there is obvious wasting, failure to thrive, extensive napkin rash, or neglected skin sores, scratches, or bruises. Admission to hospital is essential to safeguard the child and establish the diagnosis. The best evidence of trauma is radiological. Characteristically there are multiple fractures of the long bones, metaphyseal detachments, and periosteal new-bone formation.19 A full skeletal survey should be made even if the injuries seem confined to the cranium. Subdural haematoma is confirmed by needle exploration of the subdural space. It is important to exclude natural disease. Fever and leucocytosis may suggest osteitis or infantile cortical hyperostosis, but the X-ray appearances are different. Scurvy, osteogenesis imperfecta, and the bleeding diseases should be ruled out by appropriate tests. The history is rarely of value, since trauma is denied or only a trivial accident admitted. Once the diagnosis has been made, however, the parents must be plainly told the doctor’s opinion of the cause. Further direct accusation or interrogation may be harmful because it reduces the chance of getting a true picture of the parents’ social or emotional difficulties. Correct diagnosis ensures the right treatment and should lead to preventive action. If the child is returned to the parents, he may be subjected to further assaults; and the siblings may be in daneer. Violent physical assaults by parents on helpless children are rightly regarded as criminal acts. It is likely, however, that these attacks are more often the result of an unrestrained impulsive outburst than of systematic persecution. Uncertainty of the underlying causes of the assault raises doubts about the wisdom of allowing the law to deal with the problem. The courts rightly insist that evidence of guilt must be beyond reasonable doubt before parents may be deprived of custodial care or convicted of assault. If the medical evidence is insufficient or is successfully challenged the parents may not be prosecuted or they may be acquitted, and the then legally " innocent " parents may refuse help or supervision. The outcome is hardly more satisfactory if the parents are charged and found guilty. A long prison sentence may appease society’s rage, but it also breaks up the family, imposes further social and economic problems, and does little to reform the offender. Yet lenient sentences, designed to ensure the acceptance of social supervision, are often misunderstood and fail to deter. Whatever the results of prosecution, it does not help us to understand the real causes of cruelty to children, and it is in this understanding that hopes for
prevention
lie.
The " battered baby " is only one of many manifestations of parental failure. It is one which comes before doctors rather than social workers, but it should not be isolated from the total problem of emotional and physical maltreatment of children. Surprise and disappointment has 19.
Caffey, J. Brit. J. Radiol. 1957, 30,
225.
been expressed 20 that the problem is increasing at a time when the population has never had it so good. Apart from the fact that affluence has not yet blessed all sections of the community, it is by no means certain that poverty and poor housing are primary causes of child cruelty. Studies of the families of McHenry et a1.15 and the wider investigations of Philp 21 on " problem families " both suggest that defects in personality are more important than low standards of living.
CŒLIAC DISEASE AND MALIGNANT CHANGE
As is well known, certain lesions of the gastrointestinal be pre-malignant : ulcerative colitis, polyposis and chronic coli, gastritis may undergo malignant change in a small proportion of cases. Gough et al. 12 have recently suggested that coeliac disease and its adult equivalents should be added to this list. They described four patients, in whom there was good evidence of coeliac disease, who some years later had reticulosarcomas of the small bowel. The original diagnosis of coeliac disease rested on histological evidence of subtotal or total villous atrophy of the small bowel, together with a history of longstanding steatorrhoea and biochemical evidence of widespread small-bowel dysfunction. The reticulosis modified the clinical course of the disease, so that its tract may
development was suspected from the following features: relapse, with loss of weight and increasing diarrhoea, in a patient who had previously responded to a low-fat or a gluten-free diet; attacks of abdominal pain and fever; a raised erythrocyte-sedimentation rate; perforation or obstruction of the small bowel; and involvement of structures outside the abdominal cavity-for example, lymph-glands and skin. The long interval between onset of symptoms and the first evidence of malignancy (in some cases 20 years) together with the demonstration of typical mucosal defect suggested that the cceliac disease was the primary lesion and the reticulosis a complication. Intestinal reticulosis, however, is known to cause steatorrhoea, and since the first account in 1937 by Fairley and MacKie,23 many other reports have followed. 24-26Gough et al. suggested that in 16 of the 29 reports in the literature the reticulosis did in fact arise as a complication of pre-existing codiac disease. Their reasons were the long history and the evidence of a widespread disorder of small-bowel function shown at operation or necropsy to be associated with a localised lesion. A further case has been recorded by Spracklen.27 A patient who had coeliac disease at 14 months responded to unspecified dietary measures, and apart from episodes of diarrhoea she remained well until she was 31, when she relapsed and laparotomy disclosed a reticulosarcoma of the jejunum. Sections of bowel away from the tumour showed subtotal villous atrophy. The hypothesis that coeliac disease may be a premalignant condition is important because it emphasises again that patients should be carefully supervised. Since the reticulosis produces a distinctive clinical picture, early treatment should be possible. The reticulosis often appears as a localised and well-defined tumour; so early excision seems to offer the best hope of cure. 20. Brit. med. J. 1963, ii, 1544. 21. Philp, A. F. Family Failure. London, 1963. 22. Gough K. R., Read, A. E., Naish, J. M. Gut, 1962, 3, 232. 23. Fairley, N. H., MacKie, F. P. Brit. med. J. 1937, i, 375. 24. Sleisenger, M. H., Almy, T. P., Barr, D. P. Amer. J. Med. 1953, 25. Best, C. N., Cook, P. B. Brit. med. J. 1961, ii, 496. 26. Scudamore, H. H. Ann. intern. Med. 1961, 55, 433. 27. Spracklen, F. Proc. R. Soc. Med. 1963, 56, 167.
15, 666
compression and raised intracranial pressure. Vomiting, convulsions, changes in muscular tone, and loss of consciousness force the parents to seek medical aid. Important physical signs are retinal or subhyaloid haemorrhages on ophthalmoscopic examination. When the limbs have been damaged, the baby has painful, tender swellings of the arms and legs, with or without cutaneous bruising. His general state is often good, and this, together with the absence of a history of trauma, misleads the doctor into believing that an acute natural disease is the cause of the trouble. Suspicions will be more readily aroused if there is obvious wasting, failure to thrive, extensive napkin rash, or neglected skin sores, scratches, or bruises. Admission to hospital is essential to safeguard the child and establish the diagnosis. The best evidence of trauma is radiological. Characteristically there are multiple fractures of the long bones, metaphyseal detachments, and periosteal new-bone formation.19 A full skeletal survey should be made even if the injuries seem confined to the cranium. Subdural haematoma is confirmed by needle exploration of the subdural space. It is important to exclude natural disease. Fever and leucocytosis may suggest osteitis or infantile cortical hyperostosis, but the X-ray appearances are different. Scurvy, osteogenesis imperfecta, and the bleeding diseases should be ruled out by appropriate tests. The history is rarely of value, since trauma is denied or only a trivial accident admitted. Once the diagnosis has been made, however, the parents must be plainly told the doctor’s opinion of the cause. Further direct accusation or interrogation may be harmful because it reduces the chance of getting a true picture of the parents’ social or emotional difficulties. Correct diagnosis ensures the right treatment and should lead to preventive action. If the child is returned to the parents, he may be subjected to further assaults; and the siblings may be in daneer. Violent physical assaults by parents on helpless children are rightly regarded as criminal acts. It is likely, however, that these attacks are more often the result of an unrestrained impulsive outburst than of systematic persecution. Uncertainty of the underlying causes of the assault raises doubts about the wisdom of allowing the law to deal with the problem. The courts rightly insist that evidence of guilt must be beyond reasonable doubt before parents may be deprived of custodial care or convicted of assault. If the medical evidence is insufficient or is successfully challenged the parents may not be prosecuted or they may be acquitted, and the then legally " innocent " parents may refuse help or supervision. The outcome is hardly more satisfactory if the parents are charged and found guilty. A long prison sentence may appease society’s rage, but it also breaks up the family, imposes further social and economic problems, and does little to reform the offender. Yet lenient sentences, designed to ensure the acceptance of social supervision, are often misunderstood and fail to deter. Whatever the results of prosecution, it does not help us to understand the real causes of cruelty to children, and it is in this understanding that hopes for
prevention
lie.
The " battered baby " is only one of many manifestations of parental failure. It is one which comes before doctors rather than social workers, but it should not be isolated from the total problem of emotional and physical maltreatment of children. Surprise and disappointment has 19.
Caffey, J. Brit. J. Radiol. 1957, 30,
225.
been expressed 20 that the problem is increasing at a time when the population has never had it so good. Apart from the fact that affluence has not yet blessed all sections of the community, it is by no means certain that poverty and poor housing are primary causes of child cruelty. Studies of the families of McHenry et a1.15 and the wider investigations of Philp 21 on " problem families " both suggest that defects in personality are more important than low standards of living.
CŒLIAC DISEASE AND MALIGNANT CHANGE
As is well known, certain lesions of the gastrointestinal be pre-malignant : ulcerative colitis, polyposis and chronic coli, gastritis may undergo malignant change in a small proportion of cases. Gough et al. 12 have recently suggested that coeliac disease and its adult equivalents should be added to this list. They described four patients, in whom there was good evidence of coeliac disease, who some years later had reticulosarcomas of the small bowel. The original diagnosis of coeliac disease rested on histological evidence of subtotal or total villous atrophy of the small bowel, together with a history of longstanding steatorrhoea and biochemical evidence of widespread small-bowel dysfunction. The reticulosis modified the clinical course of the disease, so that its tract may
development was suspected from the following features: relapse, with loss of weight and increasing diarrhoea, in a patient who had previously responded to a low-fat or a gluten-free diet; attacks of abdominal pain and fever; a raised erythrocyte-sedimentation rate; perforation or obstruction of the small bowel; and involvement of structures outside the abdominal cavity-for example, lymph-glands and skin. The long interval between onset of symptoms and the first evidence of malignancy (in some cases 20 years) together with the demonstration of typical mucosal defect suggested that the cceliac disease was the primary lesion and the reticulosis a complication. Intestinal reticulosis, however, is known to cause steatorrhoea, and since the first account in 1937 by Fairley and MacKie,23 many other reports have followed. 24-26Gough et al. suggested that in 16 of the 29 reports in the literature the reticulosis did in fact arise as a complication of pre-existing codiac disease. Their reasons were the long history and the evidence of a widespread disorder of small-bowel function shown at operation or necropsy to be associated with a localised lesion. A further case has been recorded by Spracklen.27 A patient who had coeliac disease at 14 months responded to unspecified dietary measures, and apart from episodes of diarrhoea she remained well until she was 31, when she relapsed and laparotomy disclosed a reticulosarcoma of the jejunum. Sections of bowel away from the tumour showed subtotal villous atrophy. The hypothesis that coeliac disease may be a premalignant condition is important because it emphasises again that patients should be carefully supervised. Since the reticulosis produces a distinctive clinical picture, early treatment should be possible. The reticulosis often appears as a localised and well-defined tumour; so early excision seems to offer the best hope of cure. 20. Brit. med. J. 1963, ii, 1544. 21. Philp, A. F. Family Failure. London, 1963. 22. Gough K. R., Read, A. E., Naish, J. M. Gut, 1962, 3, 232. 23. Fairley, N. H., MacKie, F. P. Brit. med. J. 1937, i, 375. 24. Sleisenger, M. H., Almy, T. P., Barr, D. P. Amer. J. Med. 1953, 25. Best, C. N., Cook, P. B. Brit. med. J. 1961, ii, 496. 26. Scudamore, H. H. Ann. intern. Med. 1961, 55, 433. 27. Spracklen, F. Proc. R. Soc. Med. 1963, 56, 167.
15, 666